학술논문
2023 Taiwan Society of Cardiology (TSOC) and Taiwan College of Rheumatology (TCR) Joint Consensus on Connective Tissue Disease-Associated Pulmonary Arterial Hypertension
Document Type
Article
Author
Wei-Chun Huang; Song-Chou Hsieh; Yen-Wen Wu; Tsu-Yi Hsieh; Yih-Jer Wu; Ko-Jen Li; Min-Ji Charng; Wei-Sheng Chen; Shih-Hsien Sung; Yen-Po Tsao; Wan-Jing Ho; Chien-Chih Lai; Chin-Chang Cheng; Hung-Cheng Tsai; Chih-Hsin Hsu; Cheng-Hsun Lu; Yu-Wei Chiu; Chieh-Yu Shen; Chun-Hsien Wu; Feng-Cheng Liu; Yen-Hung Lin; Fu-Chiang Yeh; Wei-Shin Liu; Hui-Ting Lee; Shu-Hao Wu; Chi-Ching Chang; Chun-Yuan Chu; Charles Jia-Yin Hou; Chang-Youh Tsai
Source
Acta Cardiologica Sinica. Vol. 39 Issue 2, p213-241. 29 p.
Subject
Language
英文
ISSN
1011-6842
Abstract
Background: Pulmonary arterial hypertension (PAH), defined as the presence of a mean pulmonary artery pressure >20 mmHg, pulmonary artery wedge pressure ≤ 15 mmHg, and pulmonary vascular resistance (PVR) > 2 Wood units based on expert consensus, is characterized by a progressive and sustained increase in PVR, which may lead to right heart failure and death. PAH is a well-known complication of connective tissue diseases (CTDs), such as systemic sclerosis, systemic lupus erythematosus, Sjogren's syndrome, and other autoimmune conditions. In the past few years, tremendous progress in the understanding of PAH pathogenesis has been made, with various novel diagnostic and screening methods for the early detection of PAH proposed worldwide. Objectives: This study aimed to obtain a comprehensive understanding and provide recommendations for the management of CTD-PAH in Taiwan, focusing on its clinical importance, prognosis, risk stratification, diagnostic and screening algorithm, and pharmacological treatment. Methods: The members of the Taiwan Society of Cardiology (TSOC) and Taiwan College of Rheumatology (TCR) reviewed the related literature thoroughly and integrated clinical trial evidence and real-world clinical experience for the development of this consensus. Conclusions: Early detection by regularly screening at-risk patients with incorporations of relevant autoantibodies and biomarkers may lead to better outcomes of CTD-PAH. This consensus proposed specific screening flowcharts for different types of CTDs, the risk assessment tools applicable to the clinical scenario in Taiwan, and a recommendation of medications in the management of CTD-PAH.